- While sickle cell disease is more common in African-Americans compared to other ethnicities, it can affect people of any race or ethnicity.
- The U.S. incidence estimate for sickle cell trait is 73.1 cases per 1,000 black newborns, 6.9 cases per 1,000 Hispanic newborns, 3.0 cases per 1,000 white newborns, and 2.2 cases per 1,000 Asian or Pacific Islander newborns.
- Sickle cell disease is common in Africa, Spanish-speaking regions in South and Central America, parts of the Caribbean. Mediterranean countries (such as Greece, Turkey, and Italy), the Arabian Peninsula, and India, affecting people of all skin tones.
What Is Sickle Cell Anemia?
Sickle cell anemia is a type of sickle cell disease, an inherited disorder that affects the body’s red blood cells.
Normal red blood cells are disc-shaped and flexible in order to fit through small blood vessels. People with sickle cell disease have some red blood cells with an abnormal crescent shape that resembles a farming tool called a sickle.
These sickle cells can catch on one another or stick to blood vessel walls where they can accumulate and cause blood vessels to become blocked. Blocked blood vessels prevent the organs from getting the oxygen they need which can result in pain or organ damage.
What Are Symptoms of Sickle Cell Anemia?
Symptoms of sickle cell anemia frequently begin when a baby is a few months old and may include:
- Pain crisis, also called sickle crisis
- Can range from mild or severe and can last hours to days
- In babies
- Pain in the hands and feet with swelling and redness
- In older children and adults
- Pain in the chest
- Trouble breathing
- Fever
- Coughing
- Pain in the bones
- In the bone or joints in the shoulder or hips (called osteonecrosis or avascular necrosis)
- Severe anemia
- Lung problems
- Difficulty breathing
- Chest pain
- Asthma
- Sleep apnea
- Acute chest syndrome
- Severe and sometimes life-threatening infections
- Stroke
- Open sores on the skin (usually on the legs)
- Yellowing skin and eyes (jaundice)
- In men, a painful erection that lasts too long (priapism)
What Causes Sickle Cell Anemia?
Sickle cell anemia is caused by a genetic condition inherited from both parents. A person must inherit the sickle cell gene from both parents in order to develop sickle cell anemia.
If a person gets the sickle cell gene from just one parent, they will have other forms of sickle cell disease, or they will have “sickle cell trait,” in which a person has some minor health risks, no symptoms are present, and the gene can be passed to their children.
What Is the Treatment for Sickle Cell Anemia?
Sickle cell anemia treatment is aimed at treating symptoms and preventing organ damage and infection. Treatment for sickle cell anemia is life-long and may include:
- Adequate hydration
- Medications
- Hydroxyurea (Droxia, Hydrea)
- L-glutamine (Endari), voxelotor (Oxbryta), and crizanlizumab (Adakveo)
- Medicines to prevent and treat infections
- Daily antibiotics such as penicillin to help prevent infections in young children
- Vaccines, including a yearly flu shot, to help prevent infections
- Pain medications
- Folic acid (folate) supplementation
- Blood transfusions
- Bone marrow transplant (also called a stem cell transplant)
- This procedure can cure sickle cell disease but it’s not used regularly because it has many side effects and patients must meet certain criteria
- Gene therapy (under investigation as a potential treatment)
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https://www.uptodate.com/contents/sickle-cell-disease-the-basics?search=sickle%20ell%20anemia&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1
https://www.hopkinsmedicine.org/health/conditions-and-diseases/sickle-cell-disease
https://www.healthychildren.org/English/tips-tools/ask-the-pediatrician/Pages/Can-biracial-children-get-sickle-cell-disease.aspx
https://www.cdc.gov/ncbddd/sicklecell/features/keyfinding-trait.html